Pex13 Inactivation in the Mouse Disrupts Peroxisome Biogenesis and Leads to a Zellweger Syndrome Phenotype

The Metabolic and Molecular Bases of Inherited Disease

Scriver, R. C. (1995) · Medical Entomology and Zoology · Cited by 12,101

A cre-transgenic mouse strain for the ubiquitous deletion of loxP-flanked gene segments including deletion in germ cells

Schwenk, F., Baron, U., & Rajewsky, K. (1995) · Nucleic Acids Research · doi:10.1093/nar/23.24.5080 · Cited by 1,289

Peroxisomal and Mitochondrial Defects in the Cerebro-Hepato-Renal Syndrome

Goldfischer, S. L., Moore, C. L., Johnson, T., Spiro, A. J., Valsamis, M. P., Wisniewski, H. K., Ritch, R. H., Norton, W. T., Rapin, I., & Gartner, L. M. (1973) · Science · doi:10.1126/science.182.4107.62 · Cited by 791

A unified nomenclature for peroxisome biogenesis factors.

Distel, B., Erdmann, R., Gould, S. J., Blobel, G., Crane, D. I., Cregg, J. M., Dodt, G., Fujiki, Y., Goodman, J. M., Just, W. W., et al. (1996) · The Journal of Cell Biology · doi:10.1083/jcb.135.1.1 · Cited by 414

Peroxisome biogenesis disorders

Gould, S. J., & Valle, D. L. (2000) · Trends in Genetics · doi:10.1016/s0168-9525(00)02056-4 · Cited by 354

Peroxisome Biogenesis

Purdue, P. E., & Lazarow, P. B. (2001) · Annual Review of Cell and Developmental Biology · doi:10.1146/annurev.cellbio.17.1.701 · Cited by 323

A mouse model for Zellweger syndrome

Baes, M., Gressens, P., Baumgart, E., Carmeliet, P., Casteels, M., Fransen, M., Evrard, P., Fahimi, D., Declercq, P. E., Collen, D., et al. (1997) · Nature Genetics · doi:10.1038/ng0997-49 · Cited by 275

Pex13p is an SH3 protein of the peroxisome membrane and a docking factor for the predominantly cytoplasmic PTs1 receptor.

Gould, S. J., Kalish, J. E., Morrell, J. C., Björkman, J., Urquhart, A. J., & Crane, D. I. (1996) · The Journal of Cell Biology · doi:10.1083/jcb.135.1.85 · Cited by 243

The Human Peroxisomal Targeting Signal Receptor, Pex5p, Is Translocated into the Peroxisomal Matrix and Recycled to the Cytosol

Dammai, V., & Subramani, S. E. (2001) · Cell · doi:10.1016/s0092-8674(01)00310-5 · Cited by 241

Identification of Pex13p a peroxisomal membrane receptor for the PTS1 recognition factor.

Erdmann, R., & Blobel, G. (1996) · The Journal of Cell Biology · doi:10.1083/jcb.135.1.111 · Cited by 222

Biochemistry of mammalian peroxisomes revisited

Wanders, R. J. A., & Waterham, H. R. (2006) · Annual Review of Biochemistry · doi:10.1146/annurev.biochem.74.082803.133329 · Cited by 995

Functions and biosynthesis of plasmalogens in health and disease

Brites, P., Waterham, H. R., & Wanders, R. J. A. (2004) · Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids · doi:10.1016/j.bbalip.2003.12.010 · Cited by 430

Lipid metabolism in myelinating glial cells: lessons from human inherited disorders and mouse models

Chrast, R., Saher, G., Nave, K.-A., & Verheijen, M. H. G. (2011) · Journal of Lipid Research · doi:10.1194/jlr.r009761 · Cited by 314

The ether lipid-deficient mouse: Tracking down plasmalogen functions

Gorgas, K., Teigler, A., Komljenovic, D., & Just, W. W. (2006) · Biochimica et Biophysica Acta (BBA) - Molecular Cell Research · doi:10.1016/j.bbamcr.2006.08.038 · Cited by 235

Peroxisomal disorders I: biochemistry and genetics of peroxisome biogenesis disorders

Wanders, R. J. A., & Waterham, H. R. (2004) · Clinical Genetics · doi:10.1111/j.1399-0004.2004.00329.x · Cited by 220

Redox interplay between mitochondria and peroxisomes

Lismont, C., Nordgren, M., Van Veldhoven, P. P., & Fransen, M. (2015) · Frontiers in Cell and Developmental Biology · doi:10.3389/fcell.2015.00035 · Cited by 214

Peroxisomes in brain development and function

Berger, J., Dorninger, F., Forss-Petter, S., & Kunze, M. (2016) · Biochimica et Biophysica Acta (BBA) - Molecular Cell Research · doi:10.1016/j.bbamcr.2015.12.005 · Cited by 194

Absence of peroxisomes in mouse hepatocytes causes mitochondrial and ER abnormalities†

Dirkx, R., Vanhorebeek, I., Martens, K., Schad, A., Grabenbauer, M., Fahimi, D., Declercq, P., Van Veldhoven, P. P., & Baes, M. (2005) · Hepatology · doi:10.1002/hep.20628 · Cited by 174

Ether lipids

Magnússon, C. D., & Haraldsson, G. G. (2011) · Chemistry and Physics of Lipids · doi:10.1016/j.chemphyslip.2011.04.010 · Cited by 146

Genetic Coding Variant in GPR65 Alters Lysosomal pH and Links Lysosomal Dysfunction with Colitis Risk

Lassen, K. G., McKenzie, C. I., Mari, M. C., Murano, T., Begun, J., Baxt, L. A., Goel, G., Villablanca, E. J., Kuo, S.-Y., Huang, H., et al. (2016) · Immunity · doi:10.1016/j.immuni.2016.05.007 · Cited by 142

Be different—The diversity of peroxisomes in the animal kingdom

Islinger, M., Cardoso, M. J., & Schrader, M. (2010) · Biochimica et Biophysica Acta (BBA) - Molecular Cell Research · doi:10.1016/j.bbamcr.2010.03.013 · Cited by 122

Transcriptional coactivator PGC-1α promotes peroxisomal remodeling and biogenesis

Bagattin, A., Hugendubler, L., & Mueller, E. (2010) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1009176107 · Cited by 116

Defects in myelination, paranode organization and Purkinje cell innervation in the ether lipid-deficient mouse cerebellum

Teigler, A., Komljenovic, D., Draguhn, A., Gorgas, K., & Just, W. W. (2009) · Human Molecular Genetics · doi:10.1093/hmg/ddp110 · Cited by 99

Neocortical and cerebellar developmental abnormalities in conditions of selective elimination of peroxisomes from brain or from liver

Krysko, O., Hulshagen, L., Janssen, A., Schütz, G., Klein, R., De Bruycker, M., Espeel, M., Gressens, P., & Baes, M. (2007) · Journal of Neuroscience Research · doi:10.1002/jnr.21097 · Cited by 90

The importance of ether-phospholipids: A view from the perspective of mouse models

da Silva, T. F., Sousa, V., Malheiro, A. R., & Brites, P. (2012) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2012.05.014 · Cited by 89

Optimized protocols for the simultaneous preparation of primary neuronal cultures of the neocortex, hippocampus and cerebellum from individual newborn (P0.5) C57Bl/6J mice

Ahlemeyer, B., & Baumgart‐Vogt, E. (2005) · Journal of Neuroscience Methods · doi:10.1016/j.jneumeth.2005.05.022 · Cited by 82

Peroxisomes control mitochondrial dynamics and the mitochondrion-dependent apoptosis pathway

Tanaka, H., Okazaki, T., Aoyama-Ishiwatari, S., Yokota, M., Koike, M., Okada, Y., Fujiki, Y., & Gotoh, Y. (2019) · Journal of Cell Science · doi:10.1242/jcs.224766 · Cited by 84

Mitochondria in peroxisome-deficient hepatocytes exhibit impaired respiration, depleted DNA, and PGC-1α independent proliferation

Peeters, A., Shinde, A. B., Dirkx, R., Smet, J., Bock, K. D., Espeel, M., Vanhorebeek, I., Vanlander, A. V., Coster, R. V., Carmeliet, P., et al. (2014) · Biochimica et Biophysica Acta (BBA) - Molecular Cell Research · doi:10.1016/j.bbamcr.2014.11.017 · Cited by 85

Organelle interplay in peroxisomal disorders

Thoms, S., Grønborg, S. W., & Gärtner, J. (2009) · Trends in Molecular Medicine · doi:10.1016/j.molmed.2009.05.002 · Cited by 74

Recent insights into peroxisome biogenesis and associated diseases

Fujiki, Y., Abe, Y., Imoto, Y., Tanaka, A. J., Okumoto, K., Honsho, M., Tamura, S., Miyata, N., Yamashita, T., Chung, W. K., et al. (2020) · Journal of Cell Science · doi:10.1242/jcs.236943 · Cited by 72

Differential expression of peroxisomal matrix and membrane proteins during postnatal development of mouse brain

Ahlemeyer, B., Neubert, I., Kovacs, W. J., & Baumgart‐Vogt, E. (2007) · Journal of Comparative Neurology · doi:10.1002/cne.21448 · Cited by 68

The biogenesis protein PEX14 is an optimal marker for the identification and localization of peroxisomes in different cell types, tissues, and species in morphological studies

Grant, P. S., Ahlemeyer, B., Karnati, S., Berg, T., Stelzig, I., Nenicu, A., Kuchelmeister, K., Crane, D. I., & Baumgart‐Vogt, E. (2013) · Histochemistry and Cell Biology · doi:10.1007/s00418-013-1133-6 · Cited by 68

A New Immunomodulatory Role for Peroxisomes in Macrophages Activated by the TLR4 Ligand Lipopolysaccharide

Vijayan, V., Tumpara, S., Karnati, S., Garikapati, V., Linke, M., Kamalyan, L., Mali, S. R., Sudan, K., Kollas, A., Schmid, T., et al. (2017) · The Journal of Immunology · doi:10.4049/jimmunol.1601596 · Cited by 67

Drosophila models of peroxisomal biogenesis disorder: peroxins are required for spermatogenesis and very-long-chain fatty acid metabolism

Chen, H., Liu, Z., & Huang, X. (2009) · Human Molecular Genetics · doi:10.1093/hmg/ddp518 · Cited by 68

PEX13 deficiency in mouse brain as a model of Zellweger syndrome: abnormal cerebellum formation, reactive gliosis and oxidative stress

Müller, C. C., Nguyen, T. H., Ahlemeyer, B., Meshram, M., Santrampurwala, N., Cao, S., Sharp, P. C., Fietz, P. B., Baumgart‐Vogt, E., & Crane, D. I. (2010) · Disease Models & Mechanisms · doi:10.1242/dmm.004622 · Cited by 67

Mouse models for peroxisome biogenesis defects and β-oxidation enzyme deficiencies

Baes, M. I., & Veldhoven, P. P. V. (2012) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2012.03.003 · Cited by 70

PEX13 prevents pexophagy by regulating ubiquitinated PEX5 and peroxisomal ROS

Demers, N. D., Riccio, V., Jo, D. S., Bhandari, S., Law, K. B., Liao, W., Kim, C., McQuibban, G. A., Choe, S., Cho, D., et al. (2022) · Autophagy · doi:10.1080/15548627.2022.2160566 · Cited by 65

Hepatic dysfunction in peroxisomal disorders

Baes, M. I., & Veldhoven, P. P. V. (2015) · Biochimica et Biophysica Acta (BBA) - Molecular Cell Research · doi:10.1016/j.bbamcr.2015.09.035 · Cited by 64

Peroxisomal Stress Response and Inter-Organelle Communication in Cellular Homeostasis and Aging

Kim, J., & Bai, H. (2022) · Antioxidants · doi:10.3390/antiox11020192 · Cited by 64

The Pex1-G844D mouse: a model for mild human Zellweger spectrum disorder

Hiebler, S., Masuda, T., Hacia, J. G., Moser, A. B., Faust, P. L., Liu, A., Chowdhury, N., Huang, N., Lauer, A., Bennett, J., et al. (2014) · Molecular Genetics and Metabolism · doi:10.1016/j.ymgme.2014.01.008 · Cited by 60

[Metabolic difference between visceral fat and subcutaneous abdominal fat].

Björntorp, P. A. (2000) · PubMed · Cited by 60

Cerebral MRI as a valuable diagnostic tool in Zellweger spectrum patients

Weller, S., Rosewich, H., & Gärtner, J. (2008) · Journal of Inherited Metabolic Disease · doi:10.1007/s10545-008-0856-3 · Cited by 58

Peroxisomal protein PEX13 functions in selective autophagy

Lee, M. Y., Sumpter, R., Zou, Z., Sirasanagandla, S., Wei, Y., Mishra, P., Rosewich, H., Crane, D. I., & Levine, B. (2016) · EMBO Reports · doi:10.15252/embr.201642443 · Cited by 59

Deletion of a single allele of thePex11βgene is sufficient to cause oxidative stress, delayed differentiation and neuronal death in mouse brain

Ahlemeyer, B., Gottwald, M., & Baumgart‐Vogt, E. (2011) · Disease Models & Mechanisms · doi:10.1242/dmm.007708 · Cited by 57

Peroxisomes in cardiomyocytes and the peroxisome / peroxisome proliferator-activated receptor-loop

Colasante, C., Chen, J., Ahlemeyer, B., & Baumgart‐Vogt, E. (2015) · Thrombosis and Haemostasis · doi:10.1160/th14-06-0497 · Cited by 59

Peroxisomes Are Required for Lipid Metabolism and Muscle Function in Drosophila melanogaster

Faust, J. E., Manisundaram, A. D., Ivanova, P. T., Milne, S. B., Summerville, J. B., Brown, H. A., Wangler, M. F., Stern, M., & McNew, J. A. (2014) · PLoS ONE · doi:10.1371/journal.pone.0100213 · Cited by 54

Mendelian neurodegenerative disease genes involved in autophagy

Stamatakou, E., Wróbel, L., Hill, S. M., Puri, C., Son, S. M., Fujimaki, M., Zhu, Y., Siddiqi, F. H., Fernandez-Estévez, M., Manni, M. M., et al. (2020) · Cell Discovery · doi:10.1038/s41421-020-0158-y · Cited by 52

Revisiting the neuropathogenesis of Zellweger syndrome

Crane, D. I. (2014) · Neurochemistry International · doi:10.1016/j.neuint.2014.02.007 · Cited by 51

Failure of microtubule-mediated peroxisome division and trafficking in disorders with reduced peroxisome abundance

Nguyen, T. H., Björkman, J., Paton, B. C., & Crane, D. I. (2006) · Journal of Cell Science · doi:10.1242/jcs.02776 · Cited by 51

Peroxisome biogenesis and human peroxisome-deficiency disorders

Fujiki, Y. (2016) · Proceedings of the Japan Academy Series B · doi:10.2183/pjab.92.463 · Cited by 51

Peroxisome biogenesis disorders: Molecular basis for impaired peroxisomal membrane assembly

Fujiki, Y., Yagita, Y., & Matsuzaki, T. (2012) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2012.06.004 · Cited by 48

Molecular insights into peroxisome homeostasis and peroxisome biogenesis disorders

Fujiki, Y., Okumoto, K., Honsho, M., & Abe, Y. (2022) · Biochimica et Biophysica Acta (BBA) - Molecular Cell Research · doi:10.1016/j.bbamcr.2022.119330 · Cited by 47

Peroxisomal biogenesis is genetically and biochemically linked to carbohydrate metabolism in Drosophila and mouse

Wangler, M. F., Chao, Y.-H., Bayat, V., Γιαγτζόγλου, Ν., Shinde, A. B., Putluri, N., Coarfa, C., Donti, T. R., Graham, B. H., Faust, J. E., et al. (2017) · PLoS Genetics · doi:10.1371/journal.pgen.1006825 · Cited by 47

Drosophila Carrying Pex3 or Pex16 Mutations Are Models of Zellweger Syndrome That Reflect Its Symptoms Associated with the Absence of Peroxisomes

Nakayama, M., Sato, H., Okuda, T., Fujisawa, N., Kono, N., Arai, H., Suzuki, E., Umeda, M., Ishikawa, H., & Matsuno, K. (2011) · PLoS ONE · doi:10.1371/journal.pone.0022984 · Cited by 47

Peroxisomes and peroxisomal disorders: The main facts

Fidaleo, M. (2009) · Experimental and Toxicologic Pathology · doi:10.1016/j.etp.2009.08.008 · Cited by 45

Generalised and conditional inactivation of Pex genes in mice

Baes, M. I., & Veldhoven, P. P. V. (2006) · Biochimica et Biophysica Acta (BBA) - Molecular Cell Research · doi:10.1016/j.bbamcr.2006.08.018 · Cited by 45

Peroxisomes in mouse and human lung: their involvement in pulmonary lipid metabolism

Karnati, S., & Baumgart‐Vogt, E. (2008) · Histochemistry and Cell Biology · doi:10.1007/s00418-008-0462-3 · Cited by 42

Interstitial deletion of chromosome 2p15-16.1: Report of two patients and critical review of current genotype–phenotype correlation

Piccione, M., Piro, E., Serraino, F., Cavani, S., Ciccone, R., Malacarne, M., Pierluigi, M., Vitaloni, M., Zuffardi, O., & Corsello, G. (2012) · European Journal of Medical Genetics · doi:10.1016/j.ejmg.2012.01.014 · Cited by 38

α‐Synuclein abnormalities in mouse models of peroxisome biogenesis disorders

Yakunin, E., Moser, A. B., Loeb, V., Saada, A., Faust, P. L., Crane, D. I., Baes, M. I., & Sharon, R. (2009) · Journal of Neuroscience Research · doi:10.1002/jnr.22246 · Cited by 39

Peroxisome deficient invertebrate and vertebrate animal models

Veldhoven, P. P. V., & Baes, M. I. (2013) · Frontiers in Physiology · doi:10.3389/fphys.2013.00335 · Cited by 37

Carriers of an apolipoprotein E epsilon 4 allele are more vulnerable to a dietary deficiency in omega-3 fatty acids and cognitive decline

Nock, T. G., Chouinard‐Watkins, R., & Plourde, M. (2017) · Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids · doi:10.1016/j.bbalip.2017.07.004 · Cited by 37

Pex11a deficiency causes dyslipidaemia and obesity in mice

Chen, C., Wang, H., Chen, B., Chen, D., Lu, C., Li, H., Qian, Y., Tan, Y., Weng, H., & Cai, L. (2018) · Journal of Cellular and Molecular Medicine · doi:10.1111/jcmm.14108 · Cited by 34

Identifying candidate genes for 2p15p16.1 microdeletion syndrome using clinical, genomic, and functional analysis

Bagheri, H., Badduke, C., Qiao, Y., Colnaghi, R., Abramowicz, I., Alcantara, D., Dunham, C. P., Wen, J., Wildin, R. S., Nowaczyk, M. J. M., et al. (2016) · JCI Insight · doi:10.1172/jci.insight.85461 · Cited by 32

Overexpression of peroxisomal testis-specific 1 protein induces germ cell apoptosis and leads to infertility in male mice

Kaczmarek, K., Studencka‐Turski, M., Meinhardt, A., Wieczerzak, K., Thoms, S., Engel, W., & Grzmil, P. (2011) · Molecular Biology of the Cell · doi:10.1091/mbc.e09-12-0993 · Cited by 33

Peroxisomes in Different Skeletal Cell Types during Intramembranous and Endochondral Ossification and Their Regulation during Osteoblast Differentiation by Distinct Peroxisome Proliferator-Activated Receptors

Qian, G., Fan, W., Ahlemeyer, B., Karnati, S., & Baumgart‐Vogt, E. (2015) · PLoS ONE · doi:10.1371/journal.pone.0143439 · Cited by 32

Mitochondrial changes and oxidative stress in a mouse model of Zellweger syndrome neuropathogenesis

Rahim, R. S., Chen, M., Nourse, C. C., Meedeniya, A. C. B., & Crane, D. I. (2016) · Neuroscience · doi:10.1016/j.neuroscience.2016.08.001 · Cited by 28

Adaptor proteins: Flexible and dynamic modulators of immune cell signalling

Borowicz, P., Chan, H., Hauge, A., & Spurkland, A. (2020) · Scandinavian Journal of Immunology · doi:10.1111/sji.12951 · Cited by 33

Modulation of the hepatic fatty acid pool in peroxisomal 3-ketoacyl-CoA thiolase B-null mice exposed to the selective PPARalpha agonist Wy14,643

Arnauld, S., Fidaleo, M., Clémencet, M.-C., Chevillard, G., Athias, A., Gresti, J., Wanders, R. J. A., Latruffe, N., Nicolas-Francès, V., & Mandard, S. (2009) · Biochimie · doi:10.1016/j.biochi.2009.09.004 · Cited by 28

A defect in the peroxisomal biogenesis in germ cells induces a spermatogenic arrest at the round spermatid stage in mice

Brauns, A.-K., Heine, M., Tödter, K., Baumgart‐Vogt, E., Lüers, G. H., & Schumacher, U. (2019) · Scientific Reports · doi:10.1038/s41598-019-45991-6 · Cited by 29

Peroxisomal L-bifunctional Protein Deficiency Causes Male-specific Kidney Hypertrophy and Proximal Tubular Injury in Mice

Ranea‐Robles, P., Portman, K., Bender, A. S., Lee, K., He, C., Mulholland, D. J., Argmann, C., & Houten, S. M. (2021) · Kidney360 · doi:10.34067/kid.0003772021 · Cited by 25

Plasmalogen levels in Gaucher disease.

Moraitou, M., Dimitriou, E., Zafeiriou, D., Reppa, C., Marinakis, T., Sarafidou, J., & Michelakakis, H. (2008) · Blood Cells, Molecules & Diseases · doi:10.1016/j.bcmd.2008.03.007 · Cited by 32

Peroxisome biogenesis deficiency attenuates the BDNF-TrkB pathway-mediated development of the cerebellum

Abe, Y., Honsho, M., Itoh, R., Kawaguchi, R., Fujitani, M., Fujiwara, K., Hirokane, M., Matsuzaki, T., Nakayama, K., Ohgi, R., et al. (2018) · Life Science Alliance · doi:10.26508/lsa.201800062 · Cited by 24

Loss of functional peroxisomes leads to increased mitochondrial biogenesis and reduced autophagy that preserve mitochondrial function

Chi, L., Lee, D., Leung, S., Hu, G., Wen, B., Delgado-Olguín, P., Vissa, M., Li, R., Brumell, J. H., Kim, P. K., et al. (2023) · Cellular and Molecular Life Sciences · doi:10.1007/s00018-023-04827-3 · Cited by 23

Mammalian pexophagy at a glance

Bajdzienko, J., & Bremm, A. (2024) · Journal of Cell Science · doi:10.1242/jcs.259775 · Cited by 28

Peroxisome-Deficiency and HIF-2α Signaling Are Negative Regulators of Ketohexokinase Expression

Eberhart, T., Schönenberger, M. J., Walter, K., Charles, K. N., Faust, P. L., & Kovacs, W. J. (2020) · Frontiers in Cell and Developmental Biology · doi:10.3389/fcell.2020.00566 · Cited by 21

Zellweger syndrome caused by PEX13 deficiency: Report of two novel mutations

Al-Dirbashi, O. Y., Shaheen, R., Alsayed, M. D., Aldosari, M., Makhseed, N., Abu‐Safieh, L., Santa, T., Meyer, B. F., Shimozawa, N., & Alkuraya, F. S. (2009) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.32874 · Cited by 18

Liver disease predominates in a mouse model for mild human Zellweger spectrum disorder

Berendse, K., Boek, M., Gijbels, M. J., van der Wel, N. N., Klouwer, F. C. C., Weerman, M. A. V. D. B., Shinde, A. B., Ofman, R., Poll‐The, B. T., Houten, S. M., et al. (2019) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2019.06.013 · Cited by 17

Zebrafish model of human Zellweger syndrome reveals organ-specific accumulation of distinct fatty acid species and widespread gene expression changes

Takashima, S., Takemoto, S., Toyoshi, K., Ohba, A., & Shimozawa, N. (2021) · Molecular Genetics and Metabolism · doi:10.1016/j.ymgme.2021.05.002 · Cited by 15

Longitudinal study of Pex1-G844D NMRI mouse model: A robust pre-clinical model for mild Zellweger spectrum disorder

Demaret, T., Roumain, M., Ambroise, J., Evraerts, J., Ravau, J., Bouzin, C., Bearzatto, B., Gala, J., Stepman, H. C. M., Marie, S., et al. (2020) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2020.165900 · Cited by 15

A peroxisome deficiency–induced reductive cytosol state up-regulates the brain-derived neurotrophic factor pathway

Abe, Y., Honsho, M., Kawaguchi, R., Matsuzaki, T., Ichiki, Y., Fujitani, M., Fujiwara, K., Hirokane, M., Oku, M., Sakai, Y., et al. (2020) · Journal of Biological Chemistry · doi:10.1074/jbc.ra119.011989 · Cited by 15

Central serotonergic neuron deficiency in a mouse model of Zellweger syndrome

Rahim, R. S., Meedeniya, A. C. B., & Crane, D. I. (2014) · Neuroscience · doi:10.1016/j.neuroscience.2014.05.034 · Cited by 14

Vesicular trafficking and cell-cell communication in neurodevelopment and neurodegeneration

Amin, S., & Taverna, E. (2025) · Frontiers in Cell and Developmental Biology · doi:10.3389/fcell.2025.1600034 · Cited by 14

PEX13 is required for thermogenesis of white adipose tissue in cold-exposed mice

Park, W. Y., Park, J., Lee, S., Song, G., Nam, I.-K., Ahn, K. S., Choe, S., & Um, J. (2021) · Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids · doi:10.1016/j.bbalip.2021.159046 · Cited by 13

Peroxisomes in dental tissues of the mouse

Stelzig, I., Karnati, S., Valerius, K. P., & Baumgart‐Vogt, E. (2013) · Histochemistry and Cell Biology · doi:10.1007/s00418-013-1131-8 · Cited by 13

Peroxisomes in zebrafish: distribution pattern and knockdown studies

Krysko, O., Stevens, M. G. H., Langenberg, T., Fransen, M., Espeel, M., & Baes, M. I. (2010) · Histochemistry and Cell Biology · doi:10.1007/s00418-010-0712-z · Cited by 13

Modulation of peroxisomal import by the PEX13 SH3 domain and a proximal FxxxF binding motif

Gaussmann, S., Peschel, R., Ott, J., Żak, K. M., Sastre, J., Delhommel, F., Popowicz, G. M., Boekhoven, J., Schliebs, W., Erdmann, R., et al. (2024) · Nature Communications · doi:10.1038/s41467-024-47605-w · Cited by 13

A Transcriptomic Model of Postnatal Cardiac Effects of Prenatal Maternal Cortisol Excess in Sheep

Antolic, A. T., Richards, E. M., Wood, C. E., & KELLER-WOOD, M. E. (2019) · Frontiers in Physiology · doi:10.3389/fphys.2019.00816 · Cited by 11

Peroxisome Biogenesis Disorders

Fujiki, Y. (2006) · Encyclopedia of Life Sciences · doi:10.1038/npg.els.0006109 · Cited by 10

Peroxisome Deficiency Impairs BDNF Signaling and Memory

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